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Bethesda, MD – A new study indicates that mutant Cu/Zn superoxide dismutase (SOD1) enzymes that are associated with an inherited form of Lou Gehrig's disease cause the protein to become sticky in tissues. Partial unfolding of the mutant protein can expose hydrophobic residues that may promote abnormal interactions with other proteins or membranes in the cell. The research appears as the "Paper of the Week" in the August 19 issue of the Journal of Biological Chemistry, an American Society for Biochemistry and Molecular Biology journal. Over 5,600 people in the U.S. are diagnosed with amyotrophic lateral sclerosis (ALS) or Lou Gehrig's disease each year. About 30,000 Americans have the disease at any given time, and 10% of cases are inherited. "Amyotrophic lateral sclerosis is a neurodegenerative disorder in which neurons of the motor pathways in the brain and spinal cord die," explains Dr. Lawrence J. Hayward of the University of Massachusetts Medical School. "It typically strikes during middle age, and although it may start with only mild weakness, the symptoms can spread insidiously over months to impair mobility, speech and swallowing, and ultimately the muscles required for respiration." Despite the prevalence of ALS, the biological mechanisms that kill the motor neurons in most patients are incompletely understood. However, for a fraction of inherited ALS patients, mutations in the gene for SOD1 cause the disease by creating a toxic enzyme. Evidence suggests that misfolding or partial unfolding of mutant SOD1 proteins in these patients might be key to the toxicity.
ST. Paul, Minn. – Contrary to what you might think, most people with amyotrophic lateral sclerosis (ALS) are not depressed. They are also not more likely to get depressed as the end of life approaches, and they are not more likely to be depressed if they want to die or hasten their own death. Two new studies, published in the July 12 issue of Neurology, the scientific journal of the American Academy of Neurology, provide the evidence to contradict these assumptions. The two studies involved the same group of 80 people with advanced ALS. To enter the study, participants had to have breathing difficulties with a forced vital capacity, or breathing power, of less than 50 percent of normal, which is related to a high likelihood of hospice admission and death or the need for mechanical ventilation within six months. The participants were assessed every month until death; 53 of the participants died during the study period. The first study found that 57 percent of the participants were never depressed during the study period, and only eight percent were depressed at all visits. The researchers also found that people were not more likely to become depressed as death approached. "It's remarkable that a majority of ALS patients have a more positive attitude toward life even as the inevitability of death is imminent," Catherine Lomen-Hoerth, MD, PhD, wrote with her colleague and mentor Richard K. Olney, MD, in an accompanying editorial. Olney was founder and director of the ALS Treatment and Research Center at the University of California-San Francisco before he was diagnosed with ALS in 2004 and turned over the reins to Lomen-Hoerth, his former student.
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 16: Psychopathology: Biological Basis of Behavior Disorders
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 12: Psychopathology: Biological Basis of Behavioral Disorders
Link ID: 7629 - Posted: 06.24.2010
Proteomic signature for ALS identified MILAN, Italy, – Detection of protein abnormalities in cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS) may allow physicians to more rapidly diagnose and better monitor drug efficacy in clinical trials for the disease, according to a novel study presented by a University of Pittsburgh researcher in Milan, Italy, today. These findings may lead to the first test for early stage ALS, also know as Lou Gehrig's disease. The study, presented by Robert Bowser, Ph.D., of the University of Pittsburgh School of Medicine at the 11th annual meeting of the International Alliance of ALS/MND Associations and 14th International Symposium on ALS/MND, identified ALS-specific biomarkers by protein profiling of cerebrospinal fluid from 25 ALS patients and 35 control subjects.
– Our cells are constantly making life and death decisions. A new gene that controls this life or death switch and protects cells from dying has been discovered by researchers at the University of California, Santa Barbara, as reported in the August 28 edition of the scientific journal Nature. The discovery may provide scientists with new means for identifying drugs that combat degenerative diseases such as Lou Gehrig's disease (ALS), the destructive effects of stroke and heart diseases, autoimmune diseases, and cancer. Chilling though it sounds, our cells are poised on the brink of death. Yet the ongoing death of some of our cells is actually essential for us to live. "Death, that is to say cell death, is a key player in biology and medicine," said Joel Rothman, professor of Molecular, Cellular and Developmental Biology, and leader of the UCSB research team. "Cells often commit suicide so that others may live. This is the ultimate example of altruism at the cellular level." Copyright © 2003 Board of Regents of the University of California
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 7: Life-Span Development of the Brain and Behavior
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 13: Memory, Learning, and Development
Link ID: 4178 - Posted: 06.24.2010
St. Paul, MN –Fifty-year-old museum specimens of the Guamanian flying fox may shed more light on why Guam’s Chamorro people once had an extremely high incidence of ALS-parkinsonism dementia complex (ALS/PDC). At the same time, the study, supported by the ALS Association, introduces the role that biomagnification of neurotoxins might play in the development of symptoms similar to those of ALS, Parkinson’s disease and Alzheimer’s disease. The Chamorro people once had an incidence rate of ALS-parkinsonism dementia complex that was 50 to 100 times higher than the rate of ALS elsewhere. The decline in the incidence of ALS/PDC among the Chamorro mirrored the decline of the population of flying foxes (a type of bat), by the 1960s and 1970s. Researchers report in the August 12 issue of Neurology that skin tissues of the flying foxes contained elevated quantities of BMAA (ß-methylamino-L-alanine), a non-protein amino acid that has shown to kill neurons in cell culture, and is believed to be a possible cause of ALS/PDC.
Maybe you really are what you eat. This would solve the long-time mystery of why so many of Guam's Chamorro people – up to a third per village -- suffered a devastating neurological disease. A new study suggests that they gorged on flying fox bats that in turn had feasted on neurotoxin-laden cycad seeds. "Through the consumption of cycad-fed flying foxes, the Chamorro people may have unwittingly ingested large quantities of cycad neurotoxins," say Clark Monson of the University of Hawaii, Honolulu, Sandra Banack of California State University, Fullerton, and Paul Cox of the National Tropical Botanical Garden in Kalaheo, Hawaii, in the June issue of Conservation Biology. Guam's indigenous Chamorro people historically had a high incidence of a neurological disease with similarities to Lou Gehrig's, Parkinson's and Alzheimer's diseases. Called ALS-PDC (amyotrophic lateral sclerosis-Parkinsonian dementia complex), the disease's symptoms range from muscle weakness and paralysis to dementia. The rate of ALS-PDC has been as much as 100 times higher in Guam's Chamorro people than in the continental U.S.
ST. PAUL, MN – Researchers have discovered abnormalities in the chromosomes of several patients with sporadic, or non-hereditary, ALS, according to a study published in the April 22 issue of Neurology, the scientific journal of the American Academy of Neurology. ALS, or amyotrophic lateral sclerosis, is a progressive disease of the nervous system also known as Lou Gehrig's disease. Five to 10 percent of ALS cases are hereditary, and researchers have identified several genes that are linked to the disease. In sporadic ALS, researchers have identified several genetic risk factors for the disease, but much remains to be discovered about the role genetics play in the disease. This study examined the chromosomes of 85 people in Germany with sporadic ALS. Five people had chromosomal abnormalities, for a rate of 5.9 percent.
TROY, NY - The cause of Lou Gehrig's Disease (ALS or amyotrophic lateral sclerosis) has remained elusive since it brought down one of baseball's greatest players 60 years ago. According to Wilfredo "Freddie" Colón, ALS starts "when good proteins go bad." Understanding just why they go bad is a necessary first step toward developing medicines that will help ALS patients live with a manageable disease instead of a death sentence. The Rensselaer biochemist's vital research has recently earned a $1 million, four-year grant from the National Institutes of Health. Copyright © 1996–2001 Rensselaer Polytechnic Institute.
Results Hold Possibilities For Treating Parkinson’s and Lou Gehrig’s Disease HOUSTON, Jan. 18, 2002 – New research from University of Houston scientists may lead to techniques for jump-starting the faulty "wiring" in damaged nerve cells, and suggests possible avenues for treating spinal cord injuries, Parkinson’s disease and amyotrophic lateral sclerosis, or ALS, also known as Lou Gehrig’s disease. University of Houston scientists studying how spinal nerve cells in chicken embryos develop and function have found that chemicals called growth factors play a key role in regulating how embryonic nerve cells acquire the ability to start processing information. "In some cases, when nerves are damaged or succumb to neurodegenerative diseases such as ALS and Parkinson’s, they don’t die, but they quit working and may actually revert to an immature embryonic-like state," says Stuart Dryer, a neuroscientist in the department of biology and biochemistry at UH. Embryonic nerve cells are able to fire electrical impulses shortly after the cells have divided for the last time – after they are "born." But these impulses are extremely generic, and not necessarily specialized for the kind of information the cell is going to eventually process, Dryer says.
By Katie Moisse Sprawling blooms of cyanobacteria have swathed the surfaces of lakes and oceans around the world for billions of years. But the serene, blue-green algae may be leaching a neurotoxin into the aquatic food chain, according to a study published May 3 in Proceedings of the National Academy of Sciences ( PNAS ). The report revived a nearly 50-year-old debate over the role, if any, of the toxin in the process of neurodegeneration. In the wake of World War II a deadly neurological disease plagued the small island of Guam. The natives called it lytico-bodig (from the Spanish paralytico, meaning weakness) and it had features of Lou Gehrig's (ALS), Parkinson's and Alzheimer's diseases. Endemic to the native population (called Chamorros), the syndrome was 100 times more prevalent on Guam than anywhere else. After ruling out a genetic cause, scientists began the hunt for an environmental trigger that made Chamorros, but not immigrants, susceptible. A staple of the local cuisine raised suspicion. Chamorros made tortillas using flour ground from the seeds of cycads—plants often confused for ferns or palms and distantly related to both. The seeds were meticulously washed to remove toxins, such as beta-methylamino-L-alanine (BMAA), produced by cyanobacteria that inhabit cycad roots. Scientists wondered if BMAA could be causing neurodegeneration, but the concentrations ingested by the Chamorros were not sufficient to harm neurons in animal models. Huge concentrations, however, were. © 2010 Scientific American,
By MARIA CHENG LONDON - Lithium doesn't help patients with ALS, or Lou Gehrig's disease, contrary to previous study results, new research says. Results from a small study published two years ago suggested the drug, often used for depression, could slow the fatal neurological disorder. Many ALS sufferers and their families rushed to try it, spearheading a patient-led effort to test lithium without doctors. In the first trial to scientifically assess whether lithium works for Lou Gehrig's disease, doctors found it had no effect — and stopped the study early because it seemed futile. The results were published online Tuesday in the medical journal, Lancet Neurology. American and Canadian doctors enrolled 84 patients with Lou Gehrig's disease into their study across both countries. About half the patients got lithium and riluzole, the standard drug used for the disease. The other half got riluzole plus placebo pills. After nearly six months, researchers didn't see any difference: 22 of the 40 patients in the lithium group had gotten worse versus 20 of 44 patients in the placebo group. Patients on lithium were more likely to have fallen and suffer back pain. The study was funded by the U.S. National Institute of Neurological Disorders and Stroke, the ALS Association and the ALS Society of Canada. Copyright 2010 The Associated Press.
By MICHELE MORGAN BOLTON MIDDLEBOROUGH, Mass., — The big news in this struggling southeastern Massachusetts community is a proposed $1 billion casino complex that many hope will bring financial salvation. But for a small group of residents, the hope for economic revival is overshadowed by health concerns. They are awaiting a report later this year that could reveal whether the dozens of cases of Lou Gehrig’s disease centered around a downtown industrial area were caused by pollution. The cases, which both state and federal officials call a disease cluster, are located within a mile of Everett Square — a densely settled neighborhood adjacent to the town’s onetime factory row. It is now home to two Superfund sites. The study, which was financed by the federal Agency for Toxic Substances and Disease Registry and conducted by state health scientists, will be followed by the creation of a statewide registry to track cases of the disease, formally known as amyotrophic lateral sclerosis, the cause of which is not fully understood. State Senator Marian Walsh, a Democrat from West Roxbury, said it was understandable that most residents were more interested in the prospect of obtaining a casino, which would be built by the Mashpee Wampanoag Indians and is expected to create as many as 10,000 jobs. “It’s human nature that we move toward pleasure and away from pain,” Ms. Walsh said. “But here, if we can understand the genesis, the registry will bring in money, information and resources that will help get to a cure.” Copyright 2007 The New York Times Company
Heidi Ledford Knocking out a single gene nearly doubles the lifespan of mice with the animal model of Lou Gehrig's disease, suggesting that the gene may one day become a target for therapies in humans. Lou Gehrig's disease, otherwise known as amyotrophic lateral sclerosis (ALS), is a neurodegenerative disease that gradually erodes motor control. Death usually follows within three to five years of diagnosis. There is no cure, and the only drug available that slows progression of the disease, riluzole, prolongs survival only by a few months. Mice develop ALS-like symptoms when they have a mutation in a gene called SOD1 — a mutation that causes about 1-2% of human ALS cases. Research using these animal models has suggested that chemically reactive forms of oxygen that can damage cells also contribute to the disease. Several proteins present in the bodies of mice and people are known to generate reactive oxygen species as part of their normal function in cell signalling and inflammation. So John Engelhardt and his colleagues at the University of Iowa in Iowa City decided to look closely at two of these — Nox1 and Nox2 — to see whether turning down the amount of such proteins could slow the progression of ALS symptoms. It did — dramatically. The team found that ALS mice lacking the gene that creates Nox2 produced fewer reactive oxygen species and lived on average for 229 days — 97 days longer than those who had normal levels of Nox21. ©2007 Nature Publishing Group
Michael Hopkin British neuroscientists are planning to investigate whether playing soccer contributes to the development of motor neurone disease. The move comes after three amateur footballers playing in the same league developed the disease, which normally affects less than one person in every 50,000 each year. Experts are now aiming to launch a full epidemiological study of professional footballers and motor neurone disease (MND) patients, to see whether the sport really does raise the incidence of the disease among those who play it at a high level. Details of the patients, all of whom were committed footballers, are published in the journal Amyotrophic Lateral Sclerosis1 — a publication named after the most common form of MND. The patients range in age between 56 and 61 years old, and were all diagnosed with the disease within a decade of each other. "What is unusual about this group is that they are all friends who developed MND at the same time," says Ammar Al-Chalabi of King's College London, one of the experts who described the cases. "A cluster like this could occur by chance, but the odds are quite long." The three have several potential risk factors in common, including having been electrocuted by mains electricity at some point during their lives. But the authors note that the three were very keen at football, playing more than twice a week — almost as much as professional players. ©2007 Nature Publishing Group
In 1979 Jack O’Neil, 69, from New York City, was diagnosed with Lou Gehrig's disease, also known as ALS, or amytrophic lateral sclerosis. "The prognosis indicated that I had between two and five years to live," says O'Neil. Fortunately the disease was unusually slow to affect him, allowing him to keep working for more than 15 years. But finally in 1985 he was forced to stop working as he lost control of his muscles. "I had no energy. I couldn't fulfill my work obligations and I couldn't travel. I was just exhausted all the time," O'Neil remembers. "I could walk a little bit, but gradually I didn't have strength to do anything—to move my hands or my arms or anything like that." ALS, a rapidly progressive, invariably fatal neurological disease, is one of the most common neuromuscular diseases worldwide. The specialized nerve cells of the spine that control muscle movements in the body selectively die, leading to progressive paralysis. It strikes people mostly between 40 and 60 years of age, but sometimes even younger, and typically more men than women. As many as 20,000 Americans have ALS and around 5,000 Americans are diagnosed each year. © ScienCentral, 2000- 2004.
— The life or death of motor neurons in patients afflicted with amyotrophic lateral sclerosis (ALS) may rest with a somewhat overlooked group of support cells that helps guide, nourish and remove toxins from neurons. Howard Hughes Medical Institute researchers and their colleagues have discovered that non-neuronal cells, called astrocytes and glia, can protect neurons containing ALS-causing mutations from degeneration. Their studies also show that if non-neuronal cells harbor ALS mutations, then damage can occur in neighboring motor neurons that are otherwise healthy. According to the researchers, their findings suggest that it may be possible to insert healthy astrocytes into ALS patients to reduce or prevent motor-neuron degeneration. Motor neurons control muscle action, and they are progressively weakened in ALS, leading to paralysis and death. ©2003 Howard Hughes Medical Institute
La Jolla, Calif.—Manufacturing motor nerve cells may someday be possible to help restore function in victims of spinal cord injury or such diseases of motion as Parkinson’s and Lou Gehrig’s disease or post-polio syndrome, a Salk Institute research study has found. Salk Associate Professor Sam Pfaff and postdoctoral fellow Soo-Kyung Lee reported in a paper in the June 5 issue of Neuron that they constructed a detailed model of how stem cells are prodded, regulated and otherwise encouraged to become not only nerve cells, but specifically motor neurons that the body relies on to move muscles and limbs throughout the body. The study provides the first blueprint for the cellular factory that produces motor neurons from embryonic stem cells. It could eventually result in new treatments for spinal cord injury, and other diseases that affect motor nerve cells.
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 7: Life-Span Development of the Brain and Behavior
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 13: Memory, Learning, and Development
Link ID: 3890 - Posted: 06.24.2010
Eating bat meat may be to blame for mystery illness ASSOCIATED PRESS WASHINGTON, — Scientists have long sought to understand a horrific brain disease that once devastated the native people of Guam — Lou Gehrig’s, Alzheimer’s and Parkinson’s symptoms rolled into one. Now two researchers have uncovered clues that suggest a Chamorro dietary tradition — eating a type of bat that feeds on neurotoxic plants — might be behind the mystery illness. IT’S CIRCUMSTANTIAL evidence so far. But if the new theory is proved right, it could be more than another dismal discovery that diet can wreck the human brain. Understanding the Guam disease may help uncover novel ways to treat regular Lou Gehrig’s, Alzheimer’s or Parkinson’s diseases. The theory, published Monday in the journal Neurology, turns on the principle that changing economies can impact disease. The brain disease peaked after World War II brought guns and cash to Guam, spurring commercial hunting until the bats neared extinction — and then the human disease in turn rapidly waned, said ethnobotanist Paul Alan Cox, who studies how indigenous people relate to their environment. • MSNBC Terms, Conditions and Privacy © 2002
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 4: The Chemistry of Behavior: Neurotransmitters and Neuropharmacology
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 4: The Chemistry of Behavior: Neurotransmitters and Neuropharmacology
Link ID: 1754 - Posted: 06.24.2010
By GEOFF DAVIES A Dalhousie University professor and an international team of researchers have discovered what makes us kick. Dr. Rob Brownstone, along with colleagues in New York and Scotland, discovered a group of nerve cells that are critical to regulating how much force muscles use when performing movements. "We knew that they had to be there," Dr. Brownstone said Friday, roughly a week after Neuron, the world’s leading neuroscience journal, published the findings. "But we couldn’t pinpoint them and we couldn’t say exactly what their role was in a behaving animal." The researchers located a group of cells that regulate how much force is used by motor neurons, nerve cells in the spinal cord that make muscles contract. The team used genetic techniques to locate and deactivate these new-found "modulatory" cells in mice. "When we did that, that’s when we found that the animals couldn’t contract their muscles in their legs as much as they needed to swim properly," Dr. Brownstone said. "This is a fundamental discovery about how the spinal cord works to produce movement." Further on down the line, this discovery could lead to breakthroughs in the treatment of Lou Gehrig’s disease, spinal cord injuries and other conditions, Dr. Brownstone said. © 2009 The Halifax Herald Limited
By Terry J. Allen Researchers investigating a deadly disease cluster near a New Hampshire lake are tracking clues that stretch from a delicacy eaten on Guam to a 3.5 billion-year-old type of bacteria and the green scum that coats many New England waters. The scum - blooms of cyanobacteria often misnamed blue-green algae - produces a toxin that doctors at Dartmouth-Hitchcock Medical Center in Lebanon, N.H., suspect might have triggered cases of amyotrophic lateral sclerosis along the north shore of nearby Mascoma Lake. Using patient records and mapping software, the Dartmouth-Hitchcock team looked for ALS clusters in Maine, New Hampshire, and Vermont. Their preliminary data suggest that the disease, also known as Lou Gehrig’s disease, is about 2.5 times more prevalent among people who live within a half-mile of water bodies with past or current cyanobacteria colonies. The incidence of ALS was highest near Mascoma Lake, where nine patients have been diagnosed since 1990, all but one since 2000 - a rate at least 10 times the US average of two in 100,000 people diagnosed annually. The neurodegenerative disease eventually immobilizes patients and, inevitably, destroys their ability to swallow and breathe. In one survey, doctors said ALS is the diagnosis they most dread giving. © 2009 NY Times Co.